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Nephropathy is a common under-recognised complication of sickle cell disease (SCD) and one of the main factors of poor prognosis in these patients. The association between nephrotic syndrome and SCD in children is rare. Strategies for sickle cell nephropathy prevention are still poorly established. Blood pressure control as well as monitoring of microalbuminuria and renal function are mandatory. The use of antiproteinuric drugs, such as anti-ACE inhibitors (ACEis) and hydroxyurea, should be considered in early stages. Here, we report a case of a female adolescent with SCD and inaugural nephrotic syndrome who, after an initial treatment failure with corticotherapy, had a remarkable recovery after treatment with hydroxyurea and ACEis. © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.


Margarida Cunha, Carla Simão, Anabela Ferrão, Maria João Palaré. Nephrotic syndrome on sickle cell disease: the impact of Hydroxyurea. BMJ case reports. 2021 Mar 05;14(3)

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PMID: 33674289

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